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Liver transplantation at the Mayo Clinic.

A liver transplantation program was begun at the Mayo Clinic early in 1985. Between March and November of that year, 19 liver transplantation procedures were done in 17 patients whose ages ranged from 9 to 54 years. The preoperative conditions in these patients were primary biliary cirrhosis in eight, primary sclerosing cholangitis in four, chronic active hepatitis in three, and biliary atresia and bile duct tumor in one each. The most frequent complication of the transplantation procedure was cytomegalovirus infection, which occurred in six patients but caused moderate to severe systemic symptoms in only two of them. Of the 17 patients who underwent liver transplantation, 15 are alive. Hepatologists have determined predictive factors for survival in potential candidates in order to improve the timing of the transplantation procedure.

Adolescent↗

Results of interposition "H" grafts for portal hypertension.

During the past nine years, 54 patients underwent interposition graft shunting for variceal bleeding in 48 and intractable ascites in six, either electively (35 instances) or as an emergency (13 instances). Autogenous jugular was used in 41 instances, homologous vena cava in nine and Dacron in five. The interposition graft was placed between superior mesenteric vein and vena cava in 36 instances and the portal vein and vena cava in 19. Using Child's Clinical Classification 44 were Class C and 10 Class B. There were six (11%) operative deaths with one (2.4%) in the elective and five (38%) in the emergency group. Encephalopathy was seen in 4 (10%) of those surviving more than one year. Two Dacron and two homografts thrombosed. Eight of the autografts were patent at autopsy, 18 on angiography and 15 assumed to be patent because patients were asymptomatic. Only one of 12 late deaths was related to grafts failure. Apparently the operation controls ascites and the autogenous jugular vein is the ideal material. Interposition "H" grafting is a simple safe procedure that can be used for portal decompression in patients with bleeding varices.

Adult↗

Double-stranded DNA-binding capacity of serum in acute and chronic liver disease.

Serum antibodies to double-stranded 'native' DNA have been measured in acute and chronic liver diseases using the Farr technique. Elevated levels of DNA binding were found in all groups of patients, with the highest levels in acute viral hepatitis and lowest in primary biliary cirrhosis. All patients with hepatitis B surface antigen-positive chronic active hepatitis had elevated levels, hence persistent elevation of DNA binding after acute type B hepatitis might be an unfavourable prognostic marker indicating progression to chronic active hepatitis, DNA antibody levels will not offer diagnostic help in liver diseases, or help to follow the response of patients with 'lupoid' hepatitis to corticosteroid therapy. Production of DNA antibody may be a response to release of DNA from damaged hepatocytes.

Alcoholism↗

Thrombectomy before hepatic resection for hepatocellular carcinoma with a tumor thrombus extending to the inferior vena cava.

Tumor thrombi of hepatocellular carcinoma occasionally invade into the inferior vena cava (IVC) through the hepatic vein. Once the tumor thrombus is dislodged, severe and lethal complications, such as pulmonary infarction, can develop. We successfully operated on a hepatocellular carcinoma (HCC) patient with a tumor thrombus extending to the IVC through the right hepatic vein. To avoid dislodging the thrombus during surgery, a thrombectomy using selective hepatic vascular exclusion was performed before a hepatic resection, which is the most dangerous procedure to dislodge the thrombus.

Carcinoma, Hepatocellular↗

[Comparison of percutaneous microwave coagulation area under interruption of hepatic arterial blood flow with that under hepatic arterial and venous blood flow for hepatocellular carcinoma].

The study subjects were 49 patients with hepatocellular carcinoma. Twenty-nine patients underwent hepatic arterial blood flow block via thrombosis of the cancer-bearing area with a gelatin sponge, while the other 20 patients underwent a combination of hepatic arterial block and partial blood flow block of the portal vein with a hepatic venous balloon. Percutaneous microwave coagulation therapy (PMCT) was performed on these patients according to the type of hepatic blood flow block, and the diameters of the coagulation areas in the two groups were compared using CT images taken under portal venography. The 29 patients who underwent hepatic arterial block (A-PMCT) had a mean tumor diameter of 19.2 +/- 8 mm, while the 20 patients who underwent hepatic arterial and hepatic venous block (AV-PMCT) had a mean tumor diameter of 25.3 +/- 8.3 mm. PMCT was performed at 60 W. The mean coagulation time and the mean coagulation diameter were 4.2 +/- 1.1 min and 36.3 +/- 12.3 mm, respectively, for the A-PMCT group and 5.5 +/- 1.8 min and 43.9 +/- 9.8 mm, respectively, for the AV-PMCT group. A comparison of the coagulation diameters indicated that the AV-PMCT group had a significantly greater coagulation area than the A-PMCT group (p < 0.05).

Carcinoma, Hepatocellular↗

[A case of successful treatment of advanced hepatocellular carcinoma with tumor thrombi in the major portal branches and inferior vena cava with combined intraarterial 5-fluorouracil, adriamycin and cisplatin therapy].

The patient is a 75-year-old male. Abdominal ultrasound tomography in June 2002 revealed hepatocellular carcinoma with intrahepatic metastasis (IM3) and tumor thrombi (Vp4, Vv3) at S4 in the major portal and hepatic vein. From July 2002, he received hepatic arterial infusion therapy (FAP: 5-fluorouracil, CDDP and adriamycin) for these lesions. In December 2002, these lesions had disappeared completely after 6 sessions of arterial infusion therapy. The patient is still alive with no recurrence after 2 years since the beginning of this treatment. Recently, we treated 9 patients with combined arterial infusion chemotherapy (FAP), and the response rate (CR and PR) was 44% and no major side effect was observed. In conclusion, some patients may obtain longer survival through this treatment, even in cases of advanced HCC with tumor thrombus in the major trunks of portal vein and/or hepatic vein.

Aged↗

[Post partum occlusion of the hepatic artery: sonographic, computer tomographic and angiographic findings (author's transl)].

A case of post partum acute thrombosis of the hepatic artery with infarction of the liver is reported. The prognosis depends on the areas of obstruction, on the early development of a collateral circulation and on the contribution to the blood supply from the portal vein. The diagnosis of liver infarction was made by computed tomography. This was characterised by sharply demarkated hypodense areas, which did not enhance with contrast. An important clue is the lack of increase in density in the liver after intravenous injection of contrast when compared with the increased contrast of the spleen. This finding provided the indication for angiography. This confirmed the diagnosis of acute occlusion and made appropriate treatment possible.

Adult↗

Hepatic artery chemoembolization for advanced stage HCC: experience of 650 patients.

UNLABELLED: Hepatic artery chemotherapy using cisplatin in various protocols was examined in 650 patients. Overall objective tumor response rate (PR) was 65%. Average survival was 7.5 mo in patients with tumor progression, 18.0 mo for tumor stability and 32.0 mo for PR. 1- and 2-yr survival was 70% and 40% in responders, 20% and 0% in progressors. Prognostic factors were examined in 155 patients treated with cisplatin and gelfoam chemo-occlusion. In survival groups of > 24 mo, 4-24 mo and < 4 mo, similar numbers had cirrhosis, hepatitis B virus, hepatitis C virus and alcoholism. Decreased survival was associated with abnormal bilirubin, albumin and prothrombin time. Tumor vascularity and response to chemotherapy were associated with prolonged survival. Tumor vascularity seemed important for tumor response. Portal vein thrombosis occurred in all groups. Lesion number, bilobarity and maximum size had no correlation with response or survival. We analyzed the cause of death in 425 patients. No evidence of hepatocellular carcinoma progression, judged by absence of change in CT scan or tumor marker in the last 4 months of life, was found in 42%. A group of 57 patients were treated with cisplatin in dose range 125-200 mg/m2 alone or with gelfoam. In both groups, responders survived longer than non-responders: cisplatin alone responder mean survival, 29.0 mo, non-responder 11.1 mo, P < 0.0001. There was a strong effect of dose density on median survival for cisplatin alone, but not for cisplatin and gelfoam. CONCLUSIONS: A large experience of single-agent cisplatin chemo-occlusion is summarized. Good liver function and tumor vascularity are associated with response to chemotherapy, which in turn is associated with enhanced survival. Many deaths are due to cirrhosis and not hepatocellular carcinoma.

Antineoplastic Agents↗

Congenital hepatic fibrosis in Indian children.

BACKGROUND: Congenital hepatic fibrosis (CHF) is an uncommon cause of portal hypertension in children. So far, there is no report of this from the subcontinent. We have studied the clinical spectrum of CHF in North Indian children. METHODS: Fifteen children were diagnosed with CHF on the basis of their liver histology over a period of 6.5 years. Their clinical details were recorded. Oesophagogastroduodenoscopy and abdominal ultrasonography were performed in all cases. All siblings were examined clinically; and ultrasonography, endoscopy and liver biopsy were performed if there was firm hepatomegaly. Children with variceal bleeding were managed by endoscopic sclerotherapy. The median age of these children was 8 years with a male to female ratio of 1.5:1. RESULTS: Only one sibling (of 33) was diagnosed as having CHF. The predominant presentations were variceal bleeding in six, abdominal distension in seven and incidental detection of organomegaly in two. Hepatomegaly was present in all patients and splenomegaly in all but one. Liver function and renal function tests were normal in all children, except for a raised serum alkaline phosphatase in six. Two children had associated renal cysts, two had choledochal cysts, one each had Caroli's disease and biliary atresia and two children had portal vein thrombosis. Variceal obliteration was achieved in five children after an average 4.8 sclerotherapy sessions and one required a mesocaval shunt. On follow up (median 41 months, range 1-80 months) all are doing well. CONCLUSIONS: Congenital hepatic fibrosis is mainly sporadic in India and associated renal lesions are uncommon. Endoscopic sclerotherapy is effective in controlling variceal bleed and the prognosis is universally good in the absence of renal diseases.

Adolescent↗

Ten-year experience in porto-caval hemitransposition for liver transplantation in the presence of portal vein thrombosis.

Porto-caval hemitransposition (PCH) in liver transplantation allows revascularization of the liver when the porto-mesenteric axis is thrombosed. We, here, review our experience over an 11-year period. A total of 23 patients underwent liver transplantation using PCH. Immunosuppression was based on tacrolimus, with sirolimus used in case of renal insufficiency. Most common diagnoses were hepatitis C, Laennec's, Budd-Chiari and cryptogenic cirrhosis. Six patients needed splenectomy prior to transplant, 5 during transplant, 1 post-transplant, 11 had no splenectomy. Overall survival was 60% at 1 year and 38% at 3 years, with 10 of 23 patients currently alive and the longest survivor at 9.3 years. Most common cause of death was sepsis/multisystem organ failure, followed by pulmonary embolism. A total of 7/23 patients experienced post-operative gastrointestinal bleeding episodes, 6/23 patients developed thrombosis of the vena cava (median 162 days post-op). Post-operative ascites was noted in almost all patients. Renal dysfunction was commonly seen even after the first month post-transplant. PCH offers a feasible option for liver transplantation in those patients with complex thrombosis of the mesenteric and portal circulation.

Adult↗

Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: etiological analysis and introduction of a new concept.

Problems in definitive diagnosis and etiology of various benign nodular hepatocellular lesions were evaluated. Of these lesions, focal nodular hyperplasia (FNH), nodular regenerative hyperplasia (NRH), nodular lesions associated with idiopathic portal hypertension (IPH), non-cirrhotic large regenerative nodules (LRN), hepatocellular adenoma (HA)-like hyperplastic nodules, and partial nodular transformation (PNT) have been suggested to be related to abnormal hepatic circulation. However, the following points are considered to need further clarification: (i) is the abnormal circulation caused by thrombosis, vasculitis, or congenital anomaly?; (ii) is thrombosis a cause or a result of congestion?; (iii) are impaired blood vessels primarily the portal veins or arteries?; (iv) how are these disorders related to various syndromes, immunological abnormalities and abnormal blood flow of other organs, which are reported to coexist with these lesions often?; and (v) how should non-typical cases, which differ from typical cases, be interpreted? In addition, a concept that may lead to solving these problems (anomalous portal tract syndrome; a hypothesis that congenital vascular anomaly is the origin of these benign nodular hepatocellular lesions) was introduced.

Focal Nodular Hyperplasia↗

Treatment of Budd-Chiari syndrome due to inferior vena cava occlusion by combined portal and vena caval decompression.

This study concerns Budd-Chiari syndrome (BCS) caused by occlusion of the subdiaphragmatic inferior vena cava (IVC). It describes the experimental and clinical evaluation of the treatment of this disorder by one-stage combined portal and vena caval decompression with a direct side-to-side portacaval shunt (PCS) and a caval-atrial shunt (CAS) graft. BCS was produced in rats by gradual occlusion of the suprahepatic IVC with an ameroid constrictor. When ascites and portal hypertension were established, 12 control rats survived a sham thoracolaparotomy, 16 rats survived a mesoatrial shunt, and 16 rats survived combined PCS and CAS graft. All control rats re-formed ascites and died within 2 months. Nine of 16 rats with mesoatrial shunt developed graft thrombosis, re-formed ascites, and died within 2 months. In contrast, only 2 of 16 rats that underwent combined PCS and CAS developed graft thrombosis, re-formed ascites, and died. Liver biopsies showed reversal of severe pathologic changes in rats with patent grafts. Clinical evaluation of combined PCS and CAS using a 20-mm ring-reinforced Gore-Tex graft has been undertaken in five patients with BCS and ascites, hepatosplenomegaly, intense hepatic congestion on biopsy, and angiography showing occlusion of both the IVC and hepatic veins. All five patients are alive and well 6 months to 7.5 years postoperatively with patent grafts, no ascites or need for diuretics, no encephalopathy, normal liver function, and reversal of liver pathology. It is concluded that combined PCS and CAS create a high-flow shunt that decompresses both the portal system and IVC, has a low incidence of graft thrombosis, has been consistently effective in relieving BCS caused by IVC occlusion, and appears to be superior to mesoatrial shunt.

Adult↗